PAH

Question 1

What is the brand name of Epoprostenol (IV)?

Answer 1

Flolan

Veletri

Question 2

What is the half-life of Flolan?

Answer 2

VERY SHORT!

3-5 minutes

Question 3

What is the brand name of Treprostinil (IV/ SC)?

Answer 3

Remodulin

Question 4

What is the brand name of Treprostinil (INH)?

Answer 4

Tyvaso

Question 5

What is the brand name of Treprostinil (PO)?

Answer 5

Orenitram

Question 6

What is the brand name of Iloprost (INH)?

Answer 6

Ventavis

Question 7

What are ADRs of the Synthetic Prostacyclin and Prostacyclin Analogs?

Answer 7

Flushing
Jaw Pain
Headache
Skeletal Pain
Diarrhea

Question 8

What ADRs can be seen specifically with the the parental formulations of the synthetic prostacyclin and prostacyclin analogs?

Answer 8

Hypotenstion
Thrombocytopenia
Catheter-related infection
pump malfunction
infusion site pain (especially SC)
thrombosis

Question 9

What ADRs can be seen specifically with the INH formulations?

Answer 9

Cough

throat irritation may occur with the inhaled formulation

Question 10

Which PAH agent is shown to decrease mortality in WHO functional class IV patients?

Answer 10

Epoprostenol

Question 11

Who are the parenteral prostacyclins analogs indicated for?

Answer 11

functional class III and IV

Question 12

What is the MOA of the Synthetic Prostacyclin and Prostacylin Analogs? (2)

Answer 12

1. Direct vasodilator of systemic and pulmonary vascular beds
2. also inhibit platelet

Question 13

What is the MOA of Endothelin Receptor Antagonists (ERAs)?

Answer 13

antagonism of endothelin receptors, leading to vasodilation

Question 14

What is the brand name of Bosentan?

Answer 14

Tracleer

Question 15

What is the brand name of ambrisentan?

Answer 15

Letairis

Question 16

What is the brand name of Macitentan?

Answer 16

Opsumit

Question 17

How are the endothelin receptor antagonists metabolized?

Answer 17

hepatic metabolism

Question 18

What are the ADRs of endothelin receptor antagonists?

Answer 18

Headache
flushing
Increase Liver Function Tests ( more likely w/ bosentan)
edema ( especially if >65 yo)
decrease hemoglobin/ hematocrit

Question 19

What drug category (A, B, C, D, X) are the ERAs?

Answer 19

Teratogenic ( Category X)

Question 20

What drug has a black box warning for liver injury?

Answer 20

Bosentan

Question 21

True or False

All ERAs Drugs are Oral Agents

Answer 21

True

Question 22

What drug class are only available through access programs due to teratogenicity? (REMS)

Answer 22

ERAs

Question 23

What functional classes can one use ERAs?

Answer 23

Functional Classes II through IV

Question 24

What is the MOA of Phosphodiesterase-5 inhibitors?

Answer 24

increase cellular concentration of cGMP, leading to vasorelaxation and antiproliferative effects on vascular smooth muscle cells

Question 25

what is the brand name of sildenafil?

Answer 25

Revatio

Question 26

What is the brand name of Tadafil?

Answer 26

Adcirca

Question 27

how are the PDE-5 inhibitors metabolized?

Answer 27

hepatic metabolism

Question 28

What are some ADRs of PDE-5 inhibitors?

Answer 28

``` Headache Flushing Dyspepsia Epistaxis Rash Vision Changes ```

Question 29

What functional classes can you use PDE-5 inhibitors?

Answer 29

Functional Class II through IV

Question 30

What is the MOA of Soluble Guanylate Cyclase Stimulator?

Answer 30

Senstizes soluble guanylate cyclase to endogenous Nitric Oxide and stimulates NO and soluble soluble guanylate cyclase binding; increases cGMP leading to vasodilation

Question 31

What is the brand name Riociguat?

Answer 31

Adempas

Question 32

How are guanylate cyclase stimulator metabolized?

Answer 32

hepatic metabolism

Question 33

What are the ADRs of guanylate cyclase stimulator metabolized?

Answer 33

``` hypotension dizziness headache bleeding edema dyspepsia ```

Question 34

What drug category (A, B, C, D, X) are the guanylate cyclase stimulator ?

Answer 34

Category X

Question 35

What drugs are contraindicated with guanylate cyclase stimulators?

Answer 35

PDE-5 inhibitors

Question 36

What functional classes can I give guanylate cyclase stimulators to?

Answer 36

functional classes II and III

Question 37

True or False | Soluble Guanylate Cyclase Stimulator is available through REMs program due to teratogenicity

Answer 37

True

Question 38

What is the MOA of the selective prostacyclin IP agonists?

Answer 38

activation of the IP receptor leads to smooth muscle cell vasodilation through increased cAMP as well as inhibition of proliferation and platelet aggregation

Question 39

What is the brand name of selexipag?

Answer 39

uptravi

Question 40

How is selexipag metabolized?

Answer 40

hepatic metabolism

Question 41

What are the common ADRs of selexipag?

Answer 41

``` flushing headache diarrhea nausea vomitting ```

Question 42

what are the less common ADRs of selexipag?

Answer 42

hyperthyroidism | decrease hemoglobin

Question 43

What functional class are selective prostacyclin IP agonist?

Answer 43

studied in functional class II and III

Question 44

What tests can be monitored while on prostacyclin IP agonists?

Answer 44

thyroid function tests

Question 45

What is the MOA of CCB?

Answer 45

blocks influx of extracellular calcium leading to vasodilation

Question 46

What is the brand name of nifedipine?

Answer 46

procardia

Question 47

What is the brand name of diltiazem?

Answer 47

cardizem

Question 48

What is the brand name of amlodipine?

Answer 48

norvasc

Question 49

What is the half-life of amlodipine?

Answer 49

LONG | 35hrs

Question 50

How are CCB metabolized?

Answer 50

hepatic metabolism

Question 51

What are the ADRs of CCBs?

Answer 51

``` peripheral edema headache dizziness constipation flushing ```

Question 52

Which calcium channel blocker is a negative inotrope?

Answer 52

diltiazem

Question 53

True or False | Use is independent upon positive response in vasodilator testing

Answer 53

False | its use is DEPENDENT